KNOW THIS Flashcards
S/E of b-agonists
Hypokalemia
IF: granular pattern of immune complex deposition; LM: diffuse capillary thickening
membranous GN or Diffuse Proliferative GN
IF: granular pattern of IC deposition; LM: hyper cellular glomeruli
Acute PSGN
IF: deposition of IgG, IgM, IgA, C3 in the mesangium
Acute PSGN
EM: sub endothelial humps and “tram-track” appearance
Membranoproliferative GN
Crescent formation in glomeruli
RPGN
LM: segmental sclerosis & hyalinosis
Focal Segmental glomerulosclerosis
EM: spiking if the GBM d/t electron-dense sub epithelial deposits
Membranous GN
Heparin MOA
enhances Anti-thrombin III activity, blocking thrombin
Thrombolytics MOA
activate plasmin to degrade fibrin
Warfarin MOA
inhibit epoxide reductase (recycles Vit k)
Absence of HGPRTase
Lesch-Nyhan
Aldolase B deficiency
Fructose intolerance
Cystathionine Synthase deficiency
Homocysteinuria
Galactose-1-phosphate UDT deficiency
Galactosemia
MR, HSM, cataracts
Galactosemia
Tyrosinase deficiency
Albinism
Burr cells/Echinocytes are seen w/
kidney disease (uremia) (regular spiking of RBC)
Acanthocytes/Spur cells are seen w/
abetalipoproteinemia, Liver ds (irregular RBC spiking)
Deficiency of spectrin or ankyrin
Hereditary spherocytosis
Target cells are seen w/
“THAL” Thalassemia, HbC, Asplenia, Liver Ds
Sideroblasts are seen w/
sideroblastic anemia (iron in mito)
“spike and dome” appearance
Membranous GN
“Tram-track” appliance of sub endothelial humps
Membranoproliferative GN
rate-limiting enzyme of heme synthesis
ALA synthase (glycine + succinyl-CoA -> delta-ALA)
Acute Intermittent Porphyria Sx
abd pain, red urine, polyneuropathy, psych disturbances
Acute Intermittent Porphyria - enzyme deficiency?
porphobilinogen
Acute Intermittent Porphyria - Tx
Heme & glucose (negative feedback to ALA synthase)
Porphyria Cutanea Tarda - Sx
blistering of skin, photosensitive, tea-colored urine, hypertrichosis, facial hyperpigmentation
Porphyria Cutanea Tarda - enzyme deficiency
Uroporphyrinogen decarboxylase
Lead poisoning Sx
encephalopathy, foot/wrist drop, lead lines on gingiva, abd pain, basophilic stippling
Lead poisoning Tx
EDTA/succimer, if severe -> Dimercaprol
Lead poisoning MOA
inhibits heme synthesis (blocks ALA dehydratase & Ferrochelatase)
Tumors that can cause polycythemia vera
Pheochromocytoma, Renal cell carcinoma, HCC, Hemangioblastoma
Shine-Delgarno element
downstream from AUG start codon in prokaryotes, recognizes 16S rRNA & 30S subunit
Streptomycin MOA
inhibits the initiation of protein synthesis by binding & distorting 30S ribosome structure
Fomepizol MOA
inhibits alcohol dehydrogenase
Disulfiram MOA
inhibits acetaldehyde dehydrogenase
Order of the speed of conduction w/in the heart
Fast->Slow: Purkinje -> Atrial mm. -> Venricular mm. -> AV node
Pancoast tumors often compress
brachial plexus to cause I/L shoulder pain & cervical sympathetic chain to cause Horner’s syndrome
Pancoast tumor origin
Squamous cell carcinoma or Adenocarcinoma
Tumor w/ high thymidine uptake suggest
high DNA synthesis -> high grade
Azoles MOA
inhibit the synthesis of Ergosterol by the fungal cytochrome P450 enzymes
Auer rods
Acute Myelogenous Leukemia
Causes of Vitamin B12 def
low intake, pernicious anemia, celiacs, Diphyllobothrium latum
Hexokinase v Glucokinase
Hexokinase is found in most cells (low Km, high affinity)
Glucokinase is found in hepatocytes & b-cells (high Km, high Vmax)
Pyruvate dehydrogenase cofactors
Thiamine, Lipoic acid, CoA, FAD, NAD+
Substrate & cofactor for GABA production
Glutamate + B6
Hb Barts
4 gamma globin chains -> fatal
Tx of hemochromatosis
Deferoxamine
Orotic aciduria - enzyme deficiency
UMP synthase
Orotic aciduria - Sx
megaloblastic anemia, no hyperammonemia
RLS of beta-oxidation of fatty acids
Carnitine Acyltransferase-1
Dx test for Hereditary spherocytosis
Osmotic fragility test
Paroxysmal Nocturnal Hemoglobinuria - deficiency
missing CD55 & CD59 -> complement attacks RBCs
Cold Agglutinins
IgM production d/t EBV, Mycoplasma –> RBC lysis
Osteomyelitis in Sickle Cell pt
Salmonella
Tx for Sickle Cell pt
Hydroxyurea
Warm Agglutinins
IgG production d/t Virus, Lupus, Malignancy, Congenital immune abnormality –> RBC lysis
Infectious agents that may cause brown gallstones
E. coli, Ascaris lumbricoides, O. sinensis
Enzyme released by injured hepatocytes that may cause brown gallstones
b-glucuronidase
7a-hydroxylase MOA
converts cholesterol to bile acids (reducing the chance of gallstones)
Desmolase MOA
breaks C-C bonds, plays a role in respiration and fermentation
Esophageal ulcerations in immunocompromised pt is caused by
CMV
Polyuria that resolves w/ Vasopressin
Central Diabetes Insipidus
Red urine in the morning
Paroxysmal Nocturnal Hemoglobinuria
HTN + hypokalemia + metabolic acidosis
Hyperaldosteronism
Fever + night sweats + weight loss
TB, Lymphoma
nystagmus + scanning speech + intention tremor
Multiple Sclerosis
vWF MOA
binds exposed collagen to GpIb on platelets
Platelet activation results in secretion of
ADP, PDGF, fibrinogen, serotonin, lysosomal enzymes, TBXA2, Ca2+, thrombin
Gp2b/3a MOA
platelets after activation -> binds fibrinogen
ITP MOA
Ab to Gp2b/3a -> destruction of platelets
ITP Tx
Steroids, IVIG, splenectomy
ITP Sx
microhemorrhages, nose bleeds, petechiae, inc bleeding time
TTP MOA
def in ADAMTS13 -> platelet aggregation -> thrombosis
Muscle cramps + dark urine following exercise
McArdle Disease
ADAMTS13 def
TTP: widespread thrombosis d/t vWF -> thrombocytopenia
Glanzman Thrombasthenia - defective
Gp2b/3a -> abnormal platelet aggregation -> inc bleeding time
vWF Disease - MOA
defective vWF -> inc bleeding time & prolonged PTT
vWF Tx
Desmopressin (DDAVP)
Bernard-Soulier Disease - defective ?
Gp1b -> inability to bind platelets to vWF on collagen
Bernard-Soulier Disease - Sx
inc bleeding time, enlarged platelets
DIC
widespread activation of clotting, inc bleeding time, PT, PTT, D-dimer, & low platelets & fibrin
Triad of HUS Sx
Hemolysis, renal insufficiency, thrombocytopenia
Pentad of TTP Sx
Hemolysis, renal insufficiency, thrombocytopenia, Neuro Sx, Fever
DIC causes
obstetric complication, trauma, sepsis, pancreatitis, malignancy, tranfusion
Fructose Intolerance - deficiency of
Aldolase B
Essential Fructosuria - deficiency of
Fructokinase
Classic Galactosemia - deficiency of
Galactose-1-phosphate UDP
Thyroid cells w/ optically clear cytoplasm “orphan annie nuclei”
Papillary carcinoma
Anemia w/ hypersegmented neutrophils
Megaloblastic anemia
Eczema + recurrent infections + thrombocytopenia
Wiskott-Aldrich
Hemosiderinuria + thrombosis
Paroxysmal Nocturnal Hemoglobinuria
Most common lymphoma in adults
Diffuse Large B-cell Lymphoma
Most common lymphoma in children
Lymphoblastic Lymphoma
Reed-sternberg cells are seen w/
Hodgkin Lymphoma
Lymphoma assoc w/ Long-term Celiac Ds
Intestinal T-cell
Starry sky appearance
Burkitt Lymphoma
Lymphoma assoc w/ Sjogren syndrome, Hashimoto thyroiditis, H. pylori
Marginal Cell MALToma
Focal myocardial inflammation w/ multinucleate giant cells
Aschoff bodies seen in Rheumatic Fever
Eosinophilic cytoplasmic globules near nucleus in liver
Mallory Bodies
Onion skinning of periosteum
Ewing Sarcoma
Pseudopalisading tumor arrangement
Glioblastoma
Elevated serum uric acid
gout, Lesch-Nyhan, Myeloproliferative disease
Inherited defect in tubular amino acid transport
Hartnup disease, Cystinuria
Deficiency in homogentisic acid oxidase
Alkaptonuria
Blocked degradation of branched chain amino acids
Maple Syrup urine disease
atrophic glossitis, iron-deficient anemia, esophageal webs
Plummer-Vinson Syndrome
HbH
seen in alpha-thalassemia w/ 3 allele deletions -> beta-globins form tetramers (HbH)
Lead poisoning inhibits
ferrochelatase, ALA dehydratase, rRNA degradation enzyme –> impaired heme synthesis & basophilic stippling
Sideroblastic anemia - deficiency of
ALA synthase (X-linked)
Tx for Sideroblastic anemia
pyridoxine
t(14,18)
Follicular Lymphoma (activation of Bcl2)
t(11;14)
Mantle Cell Lymphoma (activation of Cyclin D1)
Glomus tumor
sensitive tumor under nail bed originated from modified smooth muscle cells that control thermoregulation
Carbolfuschin staining
Acid-fast stain (mycobacteria, nocardia) retains stain d/t my colic acid content
Enteropeptidase activates
Trypsinogen—> Trypsin (essential for protein digestion)
t(9,22)
Philadelphia chromosome: CML bcr-abl
t(8;14)
Burkitt Lymphoma (c-myc activation)
T(15,17)
AML M3 type (retinoid acid receptor)